Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series
Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pu...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2018-07-01
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| Series: | Pulmonary Circulation |
| Online Access: | https://doi.org/10.1177/2045894018791802 |
| _version_ | 1811229014290857984 |
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| author | Nargues A. Weir Anna Conrey Denise Lewis Alem Mehari |
| author_facet | Nargues A. Weir Anna Conrey Denise Lewis Alem Mehari |
| author_sort | Nargues A. Weir |
| collection | DOAJ |
| description | Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertension. The only approved medical therapy for chronic thromboembolic pulmonary hypertension is riociguat. We report the experience, safety and tolerability of riociguat use in a series of sickle cell disease patients with chronic thromboembolic pulmonary hypertension. |
| first_indexed | 2024-04-12T10:07:19Z |
| format | Article |
| id | doaj.art-1576b6e84670450b89fcb38f50a7688b |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-12T10:07:19Z |
| publishDate | 2018-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-1576b6e84670450b89fcb38f50a7688b2022-12-22T03:37:24ZengWileyPulmonary Circulation2045-89402018-07-01810.1177/2045894018791802Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case seriesNargues A. Weir0Anna Conrey1Denise Lewis2Alem Mehari3Inova Advanced Lung Disease Program, Falls Church, USACardiovascular and Pulmonary Branch and Hematology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, USAInova Advanced Lung Disease Program, Falls Church, USAHoward University College of Medicine, Washington DC, USAAdults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertension. The only approved medical therapy for chronic thromboembolic pulmonary hypertension is riociguat. We report the experience, safety and tolerability of riociguat use in a series of sickle cell disease patients with chronic thromboembolic pulmonary hypertension.https://doi.org/10.1177/2045894018791802 |
| spellingShingle | Nargues A. Weir Anna Conrey Denise Lewis Alem Mehari Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series Pulmonary Circulation |
| title | Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series |
| title_full | Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series |
| title_fullStr | Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series |
| title_full_unstemmed | Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series |
| title_short | Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series |
| title_sort | riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension a case series |
| url | https://doi.org/10.1177/2045894018791802 |
| work_keys_str_mv | AT narguesaweir riociguatuseinsicklecellrelatedchronicthromboembolicpulmonaryhypertensionacaseseries AT annaconrey riociguatuseinsicklecellrelatedchronicthromboembolicpulmonaryhypertensionacaseseries AT deniselewis riociguatuseinsicklecellrelatedchronicthromboembolicpulmonaryhypertensionacaseseries AT alemmehari riociguatuseinsicklecellrelatedchronicthromboembolicpulmonaryhypertensionacaseseries |